Potassium currents in human myogenic cells from healthy and congenital myotonic dystrophy foetuses

Cell Mol Biol Lett. 2009;14(2):336-46. doi: 10.2478/s11658-009-0006-4. Epub 2009 Feb 4.

Abstract

The whole-cell patch clamp technique was used to record potassium currents in in vitro differentiating myoblasts isolated from healthy and myotonic dystrophy type 1 (DM1) foetuses carrying 2000 CTG repeats. The fusion of the DM1 myoblasts was reduced in comparison to that of the control cells. The dystrophic muscle cells expressed less voltage-activated K(+) (delayed rectifier and non-inactivating delayed rectifier) and inward rectifier channels than the age-matched control cells. However, the resting membrane potential was not significantly different between the control and the DM1 cells. After four days in a differentiation medium, the dystrophic cells expressed the fast-inactivating transient outward K(+) channels, which were not observed in healthy cells. We suggest that the low level of potassium currents measured in differentiated DM1 cells could be related to their impaired fusion.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Cell Membrane / physiology
  • Electrophysiological Phenomena
  • Fetus / cytology*
  • Humans
  • Myotonic Dystrophy / pathology
  • Myotonic Dystrophy / physiopathology*
  • Patch-Clamp Techniques
  • Potassium Channels, Voltage-Gated / physiology*
  • Satellite Cells, Skeletal Muscle / pathology
  • Satellite Cells, Skeletal Muscle / physiology*

Substances

  • Potassium Channels, Voltage-Gated